Nf2

Summary

Gene Symbol: Nf2
Description: neurofibromin 2
Alias: merlin, merlin, moesin-ezrin-radixin-like protein, neurofibromatosis 2, schwannomin
Species: mouse
Products:     Nf2

Top Publications

  1. Claudio J, Lutchman M, Rouleau G. Widespread but cell type-specific expression of the mouse neurofibromatosis type 2 gene. Neuroreport. 1995;6:1942-6 pubmed
    ..The Nf2 protein, schwannomin, is expressed as a single protein isoform of approximately 80 kDa in neuronal and non-neuronal tissues...
  2. Kissil J, Johnson K, Eckman M, Jacks T. Merlin phosphorylation by p21-activated kinase 2 and effects of phosphorylation on merlin localization. J Biol Chem. 2002;277:10394-9 pubmed
    The Nf2 tumor suppressor gene product merlin is related to the membrane-cytoskeleton linker proteins of the band 4.1 superfamily, including ezrin, radixin, and moesin (ERMs)...
  3. Denisenko Nehrbass N, Goutebroze L, Galvez T, Bonnon C, Stankoff B, Ezan P, et al. Association of Caspr/paranodin with tumour suppressor schwannomin/merlin and beta1 integrin in the central nervous system. J Neurochem. 2003;84:209-21 pubmed
    ..1 domains [FERM domains (four point one, ezrin, radixin, moesin)]. Schwannomin/merlin is a tumour suppressor expressed in many cell types, including in neurons, the function and partners of ..
  4. Lallemand D, Curto M, Saotome I, Giovannini M, McClatchey A. NF2 deficiency promotes tumorigenesis and metastasis by destabilizing adherens junctions. Genes Dev. 2003;17:1090-100 pubmed
    Mutation of the Neurofibromatosis 2 (NF2) tumor suppressor gene leads to cancer development in humans and mice. Recent studies suggest that Nf2 loss also contributes to tumor metastasis...
  5. Kalamarides M, Stemmer Rachamimov A, Niwa Kawakita M, Chareyre F, Taranchon E, Han Z, et al. Identification of a progenitor cell of origin capable of generating diverse meningioma histological subtypes. Oncogene. 2011;30:2333-44 pubmed publisher
    ..meningeal precursor as the cell of origin for murine meningioma, and show that neurofibromatosis type 2 (Nf2) inactivation in prostaglandin D2 synthase (PGDS) (+) primordial meningeal cells, before the formation of the three ..
  6. James M, Han S, Polizzano C, Plotkin S, Manning B, Stemmer Rachamimov A, et al. NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth. Mol Cell Biol. 2009;29:4250-61 pubmed publisher
    Inactivating mutations of the neurofibromatosis 2 (NF2) gene, NF2, result predominantly in benign neurological tumors, schwannomas and meningiomas, in humans; however, mutations in murine Nf2 lead to a broad spectrum of cancerous tumors...
  7. Houshmandi S, Emnett R, Giovannini M, Gutmann D. The neurofibromatosis 2 protein, merlin, regulates glial cell growth in an ErbB2- and Src-dependent manner. Mol Cell Biol. 2009;29:1472-86 pubmed publisher
    Individuals with the inherited cancer predisposition syndrome neurofibromatosis 2 (NF2) develop several central nervous system (CNS) malignancies, including glial cell neoplasms (ependymomas)...
  8. Manchanda P, Jones G, Lee A, Pringle D, Zhang M, Yu L, et al. Rac1 is required for Prkar1a-mediated Nf2 suppression in Schwann cell tumors. Oncogene. 2013;32:3491-9 pubmed publisher
    ..Loss of the NF2 tumor suppressor (encoding NF2, or Merlin) is associated with upregulation of the Rac1 small GTPase, which is thought to have a key role in mediating tumor ..
  9. Gautreau A, Manent J, Fievet B, Louvard D, Giovannini M, Arpin M. Mutant products of the NF2 tumor suppressor gene are degraded by the ubiquitin-proteasome pathway. J Biol Chem. 2002;277:31279-82 pubmed
    ..of the nervous system, mostly schwannomas, is caused by mutations in the NF2 tumor suppressor gene that encodes schwannomin (Sch). Here we examined NF2 pathogenetic mutations that result in misfolding of the FERM domain...

More Information

Publications85

  1. Kissil J, Wilker E, Johnson K, Eckman M, Yaffe M, Jacks T. Merlin, the product of the Nf2 tumor suppressor gene, is an inhibitor of the p21-activated kinase, Pak1. Mol Cell. 2003;12:841-9 pubmed
    The Nf2 tumor suppressor gene codes for merlin, a protein whose function has been elusive...
  2. Bosco E, Nakai Y, Hennigan R, Ratner N, Zheng Y. NF2-deficient cells depend on the Rac1-canonical Wnt signaling pathway to promote the loss of contact inhibition of proliferation. Oncogene. 2010;29:2540-9 pubmed publisher
    The neurofibromatosis type 2 (NF2) tumor suppressor gene encodes merlin, a membrane/cytoskeleton protein necessary for the maintenance of contact inhibition of growth in cells...
  3. Robanus Maandag E, Giovannini M, van der Valk M, Niwa Kawakita M, Abramowski V, Antonescu C, et al. Synergy of Nf2 and p53 mutations in development of malignant tumours of neural crest origin. Oncogene. 2004;23:6541-7 pubmed
    Previously, we have mimicked human neurofibromatosis type 2 (NF2) in conditional Nf2 mutant (P0Cre;Nf2flox2/flox2) mice. Schwannomas, characteristic for NF2, were found at low frequency in older mice...
  4. Lau Y, Murray L, Houshmandi S, Xu Y, Gutmann D, Yu Q. Merlin is a potent inhibitor of glioma growth. Cancer Res. 2008;68:5733-42 pubmed publisher
    b>Neurofibromatosis 2 (NF2) is an inherited cancer syndrome in which affected individuals develop nervous system tumors, including schwannomas, meningiomas, and ependymomas. The NF2 protein merlin (or schwannomin) is a member of the Band 4...
  5. Johnson K, Kissil J, Fry J, Jacks T. Cellular transformation by a FERM domain mutant of the Nf2 tumor suppressor gene. Oncogene. 2002;21:5990-7 pubmed
    ..The Nf2 encoded protein, merlin, shares close sequence similarity in its amino terminus to members of the band 4...
  6. Yi C, Troutman S, Fera D, Stemmer Rachamimov A, Avila J, Christian N, et al. A tight junction-associated Merlin-angiomotin complex mediates Merlin's regulation of mitogenic signaling and tumor suppressive functions. Cancer Cell. 2011;19:527-40 pubmed publisher
    The Merlin/NF2 tumor suppressor restrains cell growth and tumorigenesis by controlling contact-dependent inhibition of proliferation...
  7. Jongsma J, van Montfort E, Vooijs M, Zevenhoven J, Krimpenfort P, van der Valk M, et al. A conditional mouse model for malignant mesothelioma. Cancer Cell. 2008;13:261-71 pubmed publisher
    Malignant mesothelioma is a devastating disease that has been associated with loss of Neurofibromatosis type 2 (NF2) and genetic lesions affecting RB and P53 pathways...
  8. Cole B, Curto M, Chan A, McClatchey A. Localization to the cortical cytoskeleton is necessary for Nf2/merlin-dependent epidermal growth factor receptor silencing. Mol Cell Biol. 2008;28:1274-84 pubmed
    b>Merlin, the product of the NF2 tumor suppressor gene, is closely related to the ERM (ezrin, radixin, moesin) proteins, which provide anchorage between membrane proteins and the underlying cortical cytoskeleton; all four proteins are ..
  9. Morris Z, McClatchey A. Aberrant epithelial morphology and persistent epidermal growth factor receptor signaling in a mouse model of renal carcinoma. Proc Natl Acad Sci U S A. 2009;106:9767-72 pubmed publisher
    ..We have shown that the NF2 tumor suppressor Merlin inhibits EGFR internalization and signaling in a cell contact-dependent manner...
  10. Kalamarides M, Niwa Kawakita M, Leblois H, Abramowski V, Perricaudet M, Janin A, et al. Nf2 gene inactivation in arachnoidal cells is rate-limiting for meningioma development in the mouse. Genes Dev. 2002;16:1060-5 pubmed
    Biallelic NF2 gene inactivation is common in sporadic and in neurofibromatosis type 2 (NF2)-related meningiomas...
  11. Manchanda N, Lyubimova A, Ho H, James M, Gusella J, Ramesh N, et al. The NF2 tumor suppressor Merlin and the ERM proteins interact with N-WASP and regulate its actin polymerization function. J Biol Chem. 2005;280:12517-22 pubmed
    The function of the NF2 tumor suppressor merlin has remained elusive despite increasing evidence for its role in actin cytoskeleton reorganization...
  12. Giovannini M, Robanus Maandag E, van der Valk M, Niwa Kawakita M, Abramowski V, Goutebroze L, et al. Conditional biallelic Nf2 mutation in the mouse promotes manifestations of human neurofibromatosis type 2. Genes Dev. 2000;14:1617-30 pubmed
    Hemizygosity for the NF2 gene in humans causes a syndromic susceptibility to schwannoma development. However, Nf2 hemizygous mice do not develop schwannomas but mainly osteosarcomas...
  13. Benhamouche S, Curto M, Saotome I, Gladden A, Liu C, Giovannini M, et al. Nf2/Merlin controls progenitor homeostasis and tumorigenesis in the liver. Genes Dev. 2010;24:1718-30 pubmed publisher
    ..the corresponding Mst/Lats/Yap pathway to mammalian liver tumorigenesis, our molecular studies suggest that Merlin is not a major regulator of YAP in liver progenitors, and that the overproliferation of Nf2(-/-) liver progenitors ..
  14. Lecomte C, Andujar P, Renier A, Kheuang L, Abramowski V, Mellottee L, et al. Similar tumor suppressor gene alteration profiles in asbestos-induced murine and human mesothelioma. Cell Cycle. 2005;4:1862-9 pubmed
    ..Mutations in Nf2, p16/Cdkn2a, p19/Arf and Trp53 genes and protein expression of p15/Cdkn2b and Cdk4 were analyzed in 12 cultures ..
  15. Li W, Cooper J, Zhou L, Yang C, Erdjument Bromage H, Zagzag D, et al. Merlin/NF2 loss-driven tumorigenesis linked to CRL4(DCAF1)-mediated inhibition of the hippo pathway kinases Lats1 and 2 in the nucleus. Cancer Cell. 2014;26:48-60 pubmed publisher
    It is currently unclear whether Merlin/NF2 suppresses tumorigenesis by activating upstream components of the Hippo pathway at the plasma membrane or by inhibiting the E3 ubiquitin ligase CRL4(DCAF1) in the nucleus...
  16. Garcia C, Gutmann D. Nf2/Merlin controls spinal cord neural progenitor function in a Rac1/ErbB2-dependent manner. PLoS ONE. 2014;9:e97320 pubmed publisher
    ..We demonstrate that the Nf2 protein, merlin, negatively regulates spinal neural progenitor cell survival and glial differentiation in an ErbB2-dependent ..
  17. Bossuyt W, Chen C, Chen Q, Sudol M, McNeill H, Pan D, et al. An evolutionary shift in the regulation of the Hippo pathway between mice and flies. Oncogene. 2014;33:1218-28 pubmed publisher
    ..We conclude that fundamental differences exist in the upstream regulatory mechanisms of Hippo signaling between Drosophila and vertebrates. ..
  18. Hara T, Bianchi A, Seizinger B, Kley N. Molecular cloning and characterization of alternatively spliced transcripts of the mouse neurofibromatosis 2 gene. Cancer Res. 1994;54:330-5 pubmed
    The human neurofibromatosis 2 (NF2) gene has recently been isolated and predicted to encode a novel protein named merlin...
  19. Fleury Feith J, Lecomte C, Renier A, Matrat M, Kheuang L, Abramowski V, et al. Hemizygosity of Nf2 is associated with increased susceptibility to asbestos-induced peritoneal tumours. Oncogene. 2003;22:3799-805 pubmed
    Biallelic NF2 gene inactivation is frequently found in human malignant mesothelioma...
  20. Klein A, Ferreira N, Ben Dor S, Duan J, Hardy J, Cox T, et al. Identification of Modifier Genes in a Mouse Model of Gaucher Disease. Cell Rep. 2016;16:2546-2553 pubmed publisher
    ..Our data identify putative modifier genes that may be involved in determining GD severity, which might help elucidate phenotypic variability between patients with similar GD mutations. ..
  21. Wiederhold T, Lee M, James M, Neujahr R, Smith N, Murthy A, et al. Magicin, a novel cytoskeletal protein associates with the NF2 tumor suppressor merlin and Grb2. Oncogene. 2004;23:8815-25 pubmed
    b>Neurofibromatosis 2 (NF2) is a dominantly inherited disorder characterized by bilateral vestibular schwannomas and meningiomas...
  22. Shaw R, McClatchey A, Jacks T. Localization and functional domains of the neurofibromatosis type II tumor suppressor, merlin. Cell Growth Differ. 1998;9:287-96 pubmed
    ..The encoded protein, merlin, is closely related to ezrin, radixin, and moesin, which are members of the band 4...
  23. Peyre M, Salaud C, Clermont Taranchon E, Niwa Kawakita M, Goutagny S, Mawrin C, et al. PDGF activation in PGDS-positive arachnoid cells induces meningioma formation in mice promoting tumor progression in combination with Nf2 and Cdkn2ab loss. Oncotarget. 2015;6:32713-22 pubmed publisher
    ..We found a correlation of PDGFR-B expression and NF2 inactivation in a cohort of human meningiomas, and we showed that, in mice, Nf2 loss and PDGF over-expression in ..
  24. Chiasson MacKenzie C, Morris Z, BACA Q, Morris B, Coker J, Mirchev R, et al. NF2/Merlin mediates contact-dependent inhibition of EGFR mobility and internalization via cortical actomyosin. J Cell Biol. 2015;211:391-405 pubmed publisher
    ..We previously identified the neurofibromatosis type 2 (NF2) tumor suppressor Merlin as a critical mediator of contact-dependent inhibition of proliferation and specifically found that Merlin ..
  25. Gutmann D, Giovannini M. Mouse models of neurofibromatosis 1 and 2. Neoplasia. 2002;4:279-90 pubmed
    ..are prone to the development of astrocytomas and peripheral nerve sheath tumors whereas those affected with neurofibromatosis 2 (NF2) develop schwannomas and meningiomas...
  26. Huynh D, Nechiporuk T, Pulst S. Alternative transcripts in the mouse neurofibromatosis type 2 (NF2) gene are conserved and code for schwannomins with distinct C-terminal domains. Hum Mol Genet. 1994;3:1075-9 pubmed
    ..The NF2 gene product, schwannomin, is a member of a superfamily of proteins thought to link cytoskeletal elements to cell membrane components...
  27. Mori M, Triboulet R, Mohseni M, Schlegelmilch K, Shrestha K, Camargo F, et al. Hippo signaling regulates microprocessor and links cell-density-dependent miRNA biogenesis to cancer. Cell. 2014;156:893-906 pubmed publisher
    ..Thus, the Hippo pathway links contact-inhibition regulation to miRNA biogenesis and may be responsible for the widespread miRNA repression observed in cancer. ..
  28. Takeuchi K, Kawashima A, Nagafuchi A, Tsukita S. Structural diversity of band 4.1 superfamily members. J Cell Sci. 1994;107 ( Pt 7):1921-8 pubmed
    ..1 superfamily can be subdivided into 5 gene families; band 4.1 protein, ERM (ezrin/radixin/moesin/merlin/NBL6/NBL7+ ++), talin, PTPH1 (PTPH1/PTPMEG/NBL1-3), and NBL4 (NBL4/NBL5) families...
  29. Rangwala R, Banine F, Borg J, Sherman L. Erbin regulates mitogen-activated protein (MAP) kinase activation and MAP kinase-dependent interactions between Merlin and adherens junction protein complexes in Schwann cells. J Biol Chem. 2005;280:11790-7 pubmed
    Biallelic mutations in the neurofibromatosis 2 (NF2) gene are linked to schwannoma and meningioma tumorigenesis...
  30. Ghebranious N, Donehower L. Mouse models in tumor suppression. Oncogene. 1998;17:3385-400 pubmed
    ..In this review, we discuss some of the mechanistic insights provided by tumor suppressor-deficient mice and their utility as models for human cancer syndromes. ..
  31. Schulz A, Büttner R, Toledo A, Baader S, von Maltzahn J, Irintchev A, et al. Neuron-Specific Deletion of the Nf2 Tumor Suppressor Impairs Functional Nerve Regeneration. PLoS ONE. 2016;11:e0159718 pubmed publisher
    ..The tumor suppressor protein merlin, mutated in the hereditary tumor syndrome Neurofibromatosis type 2 (NF2), has recently been shown to have RhoA ..
  32. Garcia Rendueles M, Ricarte Filho J, Untch B, Landa I, Knauf J, Voza F, et al. NF2 Loss Promotes Oncogenic RAS-Induced Thyroid Cancers via YAP-Dependent Transactivation of RAS Proteins and Sensitizes Them to MEK Inhibition. Cancer Discov. 2015;5:1178-93 pubmed publisher
    ..The 22q tumor suppressor NF2, encoding merlin, is implicated in this interaction because of its frequent loss of function in human thyroid cancer cell lines...
  33. Makałowski W, Zhang J, Boguski M. Comparative analysis of 1196 orthologous mouse and human full-length mRNA and protein sequences. Genome Res. 1996;6:846-57 pubmed
    ..Also, they should prove useful in estimating the additional sampling diversity provided by mouse EST sequencing projects designed to complement the existing human cDNA collection. ..
  34. Li W, Cooper J, Karajannis M, Giancotti F. Merlin: a tumour suppressor with functions at the cell cortex and in the nucleus. EMBO Rep. 2012;13:204-15 pubmed
    ..The FERM domain protein Merlin, encoded by the NF2 tumour suppressor gene, is an important mediator of contact inhibition...
  35. Huynh D, Tran T, Nechiporuk T, Pulst S. Expression of neurofibromatosis 2 transcript and gene product during mouse fetal development. Cell Growth Differ. 1996;7:1551-61 pubmed
    b>Neurofibromatosis 2 (NF2) is an autosomal dominant inherited disorder that predisposes to benign tumors of the nervous system as well as a variety of ocular abnormalities...
  36. Claudio J, Malo D, Rouleau G. The mouse neurofibromatosis type 2 gene maps to chromosome 11. Genomics. 1994;21:437-9 pubmed
    Neurofibromatosis type 2 (NF2) is a dominantly inherited disease characterized by the development of bilateral vestibular schwannomas and meningiomas, which together represent 30% of primary brain tumors...
  37. Gehlhausen J, Park S, Hickox A, Shew M, Staser K, Rhodes S, et al. A murine model of neurofibromatosis type 2 that accurately phenocopies human schwannoma formation. Hum Mol Genet. 2015;24:1-8 pubmed publisher
    Neurofibromatosis type 2 (NF2) is an autosomal dominant genetic disorder resulting from germline mutations in the NF2 gene. Bilateral vestibular schwannomas, tumors on cranial nerve VIII, are pathognomonic for NF2 disease...
  38. Mani T, Hennigan R, Foster L, Conrady D, Herr A, Ip W. FERM domain phosphoinositide binding targets merlin to the membrane and is essential for its growth-suppressive function. Mol Cell Biol. 2011;31:1983-96 pubmed publisher
    The neurofibromatosis type 2 tumor suppressor protein, merlin, is related to the ERM (ezrin, radixin, and moesin) family of plasma membrane-actin cytoskeleton linkers...
  39. Gao X, Zhao Y, Stemmer Rachamimov A, Liu H, Huang P, Chin S, et al. Anti-VEGF treatment improves neurological function and augments radiation response in NF2 schwannoma model. Proc Natl Acad Sci U S A. 2015;112:14676-81 pubmed publisher
    ..Treatment with bevacizumab is associated with tumor control and hearing improvement in neurofibromatosis type 2 (NF2) patients; however, its effect is not durable and its mechanism of action on nerve function is unknown...
  40. Matsuda T, Zhai P, Sciarretta S, Zhang Y, Jeong J, Ikeda S, et al. NF2 Activates Hippo Signaling and Promotes Ischemia/Reperfusion Injury in the Heart. Circ Res. 2016;119:596-606 pubmed publisher
    b>NF2 (neurofibromin 2) is an established tumor suppressor that promotes apoptosis and inhibits growth in a variety of cell types, yet its function in cardiomyocytes remains largely unknown...
  41. Kim M, Kim M, Lee S, Kuninaka S, Saya H, Lee H, et al. cAMP/PKA signalling reinforces the LATS-YAP pathway to fully suppress YAP in response to actin cytoskeletal changes. EMBO J. 2013;32:1543-55 pubmed publisher
    ..We also implicate neurofibromin 2 as an AKAP (A-kinase-anchoring protein) scaffold protein that facilitates the function of the cAMP/PKA-LATS-YAP pathway. Our study thus incorporates PKA as novel component of the Hippo pathway. ..
  42. Peyre M, Stemmer Rachamimov A, Clermont Taranchon E, Quentin S, El Taraya N, Walczak C, et al. Meningioma progression in mice triggered by Nf2 and Cdkn2ab inactivation. Oncogene. 2013;32:4264-72 pubmed publisher
    ..two main events associated with meningioma progression: an increase of chromosomal instability in tumors with NF2 inactivation and homozygous deletions or point mutations of the CDKN2AB locus...
  43. Seroussi E, Pan H, Kedra D, Roe B, Dumanski J. Characterization of the human NIPSNAP1 gene from 22q12: a member of a novel gene family. Gene. 1998;212:13-20 pubmed
    ..Although the function of the NIPSNAP protein family is unknown, clues about its role may reside in the co-expression of the C. elegans orthologue, within an operon encoding protein motifs known to be involved in vesicular transport. ..
  44. Mindos T, Dun X, North K, Doddrell R, Schulz A, Edwards P, et al. Merlin controls the repair capacity of Schwann cells after injury by regulating Hippo/YAP activity. J Cell Biol. 2017;216:495-510 pubmed publisher
    Loss of the Merlin tumor suppressor and activation of the Hippo signaling pathway play major roles in the control of cell proliferation and tumorigenesis...
  45. Shi X, Yin Z, Ling B, Wang L, Liu C, Ruan X, et al. Rho differentially regulates the Hippo pathway by modulating the interaction between Amot and Nf2 in the blastocyst. Development. 2017;144:3957-3967 pubmed publisher
    ..Ser176, thus stabilizing the interaction between Amot and F-actin, and restricting the binding between Amot and Nf2. Moreover, Rho attenuates the interaction between Amot and Nf2 by binding to the coiled-coil domain of Amot...
  46. Larsson J, Ohishi M, Garrison B, Aspling M, Janzen V, Adams G, et al. Nf2/merlin regulates hematopoietic stem cell behavior by altering microenvironmental architecture. Cell Stem Cell. 2008;3:221-7 pubmed publisher
    ..We assessed whether the tumor suppressor and mediator of cell contact inhibition Nf2/merlin plays a role in governing the hematopoietic stem cell pool by stem cell-autonomous or niche-determined processes...
  47. Bai Y, Liu Y, Wang H, Xu Y, Stamenkovic I, Yu Q. Inhibition of the hyaluronan-CD44 interaction by merlin contributes to the tumor-suppressor activity of merlin. Oncogene. 2007;26:836-50 pubmed
    Mutation or loss of expression of merlin is responsible for neurofibromatosis type 2 (NF2), which is characterized by the development of schwannomas and other tumors of the nervous system...
  48. McClatchey A, Saotome I, Mercer K, Crowley D, Gusella J, Bronson R, et al. Mice heterozygous for a mutation at the Nf2 tumor suppressor locus develop a range of highly metastatic tumors. Genes Dev. 1998;12:1121-33 pubmed
    ..The neurofibromatosis type II (NF2) tumor suppressor gene encodes a member of the ezrin/radixin/moesin (ERM) family of membrane/cytoskeleton linker ..
  49. Shimizu T, Seto A, Maita N, Hamada K, Tsukita S, Tsukita S, et al. Structural basis for neurofibromatosis type 2. Crystal structure of the merlin FERM domain. J Biol Chem. 2002;277:10332-6 pubmed
    ..The NF2 gene product merlin is a tumor suppressor, and its mutation or inactivation causes this disease...
  50. Chen Q, Zhang N, Xie R, Wang W, Cai J, Choi K, et al. Homeostatic control of Hippo signaling activity revealed by an endogenous activating mutation in YAP. Genes Dev. 2015;29:1285-97 pubmed publisher
    ..These findings reveal a robust homeostatic mechanism that maintains physiological levels of YAP activity and caution against the assumptive use of YAP localization alone as a surrogate of YAP activity. ..
  51. Vitte J, Gao F, Coppola G, Judkins A, Giovannini M. Timing of Smarcb1 and Nf2 inactivation determines schwannoma versus rhabdoid tumor development. Nat Commun. 2017;8:300 pubmed publisher
    ..we generated different tissue- and developmental stage-specific conditional knockout mice carrying Smarcb1 and/or Nf2 deletion...
  52. Mori T, Gotoh S, Shirakawa M, Hakoshima T. Structural basis of DDB1-and-Cullin 4-associated Factor 1 (DCAF1) recognition by merlin/NF2 and its implication in tumorigenesis by CD44-mediated inhibition of merlin suppression of DCAF1 function. Genes Cells. 2014;19:603-19 pubmed publisher
    b>Merlin, a tumor suppressor encoded by the neurofibromatosis type 2 gene, has been shown to suppress tumorigenesis by inhibiting the Cullin 4-RING E3 ubiquitin ligase CRL4(DCAF) (1) in the nucleus...
  53. Moon K, Kim H, Lee D, Rao M, Levine E, Lim D, et al. Differential Expression of NF2 in Neuroepithelial Compartments Is Necessary for Mammalian Eye Development. Dev Cell. 2018;44:13-28.e3 pubmed publisher
    ..Neurofibromin 2 (Nf2) is strongly expressed in slowly expanding RPE and CM compartments, and the loss of mouse Nf2 causes hyperplasia in ..
  54. Jean D, Thomas E, Manie E, Renier A, de Reynies A, Lecomte C, et al. Syntenic relationships between genomic profiles of fiber-induced murine and human malignant mesothelioma. Am J Pathol. 2011;178:881-94 pubmed publisher
    ..Overall, these data show similarities between murine and human disease, and contribute to the understanding of the influence of fibers in the pathogenesis of mesothelioma and validation of the murine model for preclinical testing. ..
  55. Haase V, Trofatter J, MacCollin M, Tarttelin E, Gusella J, Ramesh V. The murine NF2 homologue encodes a highly conserved merlin protein with alternative forms. Hum Mol Genet. 1994;3:407-11 pubmed
    The recently isolated gene for neurofibromatosis type 2 (NF2) encodes a 595 amino acid protein, named merlin, which is related to the cytoskeleton-associated proteins moesin, ezrin and radixin...
  56. Giovannini M, Robanus Maandag E, Niwa Kawakita M, van der Valk M, Woodruff J, Goutebroze L, et al. Schwann cell hyperplasia and tumors in transgenic mice expressing a naturally occurring mutant NF2 protein. Genes Dev. 1999;13:978-86 pubmed
    ..We tested whether this mechanism may also apply for the neurofibromatosis type-2 gene (NF2) which, when mutated, leads to schwannoma development...
  57. Lallemand D, Saint Amaux A, Giovannini M. Tumor-suppression functions of merlin are independent of its role as an organizer of the actin cytoskeleton in Schwann cells. J Cell Sci. 2009;122:4141-9 pubmed publisher
    b>Merlin is the product of the Nf2 tumor-suppressor gene, and inactivation of Nf2 leads to the development of neural tumors such as schwannomas and meningiomas in humans and mice...
  58. Kalamarides M, Stemmer Rachamimov A, Takahashi M, Han Z, Chareyre F, Niwa Kawakita M, et al. Natural history of meningioma development in mice reveals: a synergy of Nf2 and p16(Ink4a) mutations. Brain Pathol. 2008;18:62-70 pubmed
    ..Biallelic inactivation of the neurofibromatosis 2 (NF2) tumor suppressor is associated with meningioma formation in all NF2 patients and 60% of sporadic ..
  59. McLaughlin M, Kruger G, Slocum K, Crowley D, Michaud N, Huang J, et al. The Nf2 tumor suppressor regulates cell-cell adhesion during tissue fusion. Proc Natl Acad Sci U S A. 2007;104:3261-6 pubmed
    ..We found that in the mouse embryo, expression of the Nf2 tumor suppressor, merlin, is dynamically regulated during tissue fusion: Nf2 expression is low at the leading front before fusion and high ..
  60. MacCollin M, Ramesh V, Jacoby L, Louis D, Rubio M, Pulaski K, et al. Mutational analysis of patients with neurofibromatosis 2. Am J Hum Genet. 1994;55:314-20 pubmed
    b>Neurofibromatosis 2 (NF2) is a genetic disorder characterized by the development of multiple nervous-system tumors in young adulthood. The NF2 gene has recently been isolated and found to encode a new member of the protein 4...
  61. Bono P, Cordero E, Johnson K, Borowsky M, Ramesh V, Jacks T, et al. Layilin, a cell surface hyaluronan receptor, interacts with merlin and radixin. Exp Cell Res. 2005;308:177-87 pubmed
    ..We have identified merlin, the neurofibromatosis type 2 tumor suppressor protein and radixin, as other interactors with the carboxy-terminal ..
  62. Cooper J, Li W, You L, Schiavon G, Pepe Caprio A, Zhou L, et al. Merlin/NF2 functions upstream of the nuclear E3 ubiquitin ligase CRL4DCAF1 to suppress oncogenic gene expression. Sci Signal. 2011;4:pt6 pubmed publisher
    ..1, ezrin, radixin, moesin) domain-containing protein Merlin, which is encoded by the NF2 (neurofibromatosis type 2) tumor suppressor gene...
  63. Yamauchi J, Miyamoto Y, Kusakawa S, Torii T, Mizutani R, Sanbe A, et al. Neurofibromatosis 2 tumor suppressor, the gene induced by valproic acid, mediates neurite outgrowth through interaction with paxillin. Exp Cell Res. 2008;314:2279-88 pubmed publisher
    ..Here, we show that VPA up-regulates the neurofibromatosis type 2 (NF2) tumor suppressor, merlin, to regulate neurite outgrowth through the interaction with paxillin...
  64. Morales F, Molina J, Hayashi Y, Georgescu M. Overexpression of ezrin inactivates NF2 tumor suppressor in glioblastoma. Neuro Oncol. 2010;12:528-39 pubmed publisher
    ..We report that the cytoskeletal-related proteins neurofibromatosis type 2 (NF2) and ezrin have opposite yet interdependent activities in glioblastoma growth...
  65. Abdel Majid R, Leong W, Schalkwyk L, Smallman D, Wong S, Storm D, et al. Loss of adenylyl cyclase I activity disrupts patterning of mouse somatosensory cortex. Nat Genet. 1998;19:289-91 pubmed
    ..These results provide the first evidence for involvement of cAMP signalling pathways in pattern formation of the brain. ..
  66. Kwan J, Sczaniecka A, Heidary Arash E, Nguyen L, Chen C, Ratkovic S, et al. DLG5 connects cell polarity and Hippo signaling protein networks by linking PAR-1 with MST1/2. Genes Dev. 2016;30:2696-2709 pubmed publisher
    ..These data reveal a direct connection between cell polarity proteins and Hippo, which is essential for proper development of multicellular organisms. ..
  67. Gladden A, Hebert A, Schneeberger E, McClatchey A. The NF2 tumor suppressor, Merlin, regulates epidermal development through the establishment of a junctional polarity complex. Dev Cell. 2010;19:727-39 pubmed publisher
    The neurofibromatosis type 2 (NF2) tumor suppressor, Merlin, is a FERM (Four point one, Ezrin, Radixin, Moesin) domain-containing protein whose loss results in defective morphogenesis and tumorigenesis in multiple tissues...
  68. Ahmad I, Fernando A, Gurgel R, Jason Clark J, Xu L, Hansen M. Merlin status regulates p75(NTR) expression and apoptotic signaling in Schwann cells following nerve injury. Neurobiol Dis. 2015;82:114-122 pubmed publisher
    ..b>Merlin is the protein product of the NF2 tumor suppressor gene implicated in SC tumorigenesis...
  69. Abbassi L, Malki S, Cockburn K, Macaulay A, Robert C, Rossant J, et al. Multiple Mechanisms Cooperate to Constitutively Exclude the Transcriptional Co-Activator YAP from the Nucleus During Murine Oogenesis. Biol Reprod. 2016;94:102 pubmed publisher
    ..We conclude that nuclear YAP does not play a significant physiological role during oocyte development in mammals. ..
  70. Shollar D, Del Valle L, Khalili K, Otte J, Gordon J. JCV T-antigen interacts with the neurofibromatosis type 2 gene product in a transgenic mouse model of malignant peripheral nerve sheath tumors. Oncogene. 2004;23:5459-67 pubmed
    ..immunohistochemistry and immunoprecipitation/Western blotting, but not in normal tissues and was colocalized with NF2, the putative tumor suppressor protein associated with neurofibromatosis type 2, in the nucleus of some cells...
  71. Wiley L, Dattilo L, Kang K, Giovannini M, Beebe D. The tumor suppressor merlin is required for cell cycle exit, terminal differentiation, and cell polarity in the developing murine lens. Invest Ophthalmol Vis Sci. 2010;51:3611-8 pubmed publisher
    PURPOSE. Neurofibromatosis type 2 (NF2) is an autosomal-dominant CNS tumor syndrome that affects 1:25,000 children and young adults. More than 50% of NF2 patients also develop posterior subcapsular cataracts (PSCs)...
  72. Serinagaoglu Y, Paré J, Giovannini M, Cao X. Nf2-Yap signaling controls the expansion of DRG progenitors and glia during DRG development. Dev Biol. 2015;398:97-109 pubmed publisher
    ..We further show that the Neurofibromatosis 2 (Nf2) tumor suppressor inhibits Yap during DRG development...
  73. Jin H, Sperka T, Herrlich P, Morrison H. Tumorigenic transformation by CPI-17 through inhibition of a merlin phosphatase. Nature. 2006;442:576-9 pubmed
    The tumour suppressor protein merlin (encoded by the neurofibromatosis type 2 gene NF2) is an important regulator of proliferation in many cell and tissue types...
  74. Lim J, Kim H, Jeun S, Kang S, Lee K. Merlin inhibits growth hormone-regulated Raf-ERKs pathways by binding to Grb2 protein. Biochem Biophys Res Commun. 2006;340:1151-7 pubmed
    Numerous studies have suggested that the NF2 protein merlin is involved in the regulation of abnormal cell growth and proliferation...
  75. Lendahl U. A growing family of Notch ligands. Bioessays. 1998;20:103-7 pubmed
    ..Taken together, these reports extend our knowledge about an important event in cell-cell communication--how Notch ligands and receptors meet and interact. ..
  76. Yi C, Shen Z, Stemmer Rachamimov A, Dawany N, Troutman S, Showe L, et al. The p130 isoform of angiomotin is required for Yap-mediated hepatic epithelial cell proliferation and tumorigenesis. Sci Signal. 2013;6:ra77 pubmed publisher
    ..and tumorigenesis in response to toxin-induced injury or when crossed with mice lacking the tumor suppressor Nf2. Biochemical examination of the Amot-Yap interaction revealed that the p130 splicing isoform of Amot (Amot-p130) ..